5613512 ASSESSMENT OF CHRONIC PAIN IN CHILDREN AND ADOLESCENTS WITH SICKLE CELL DISEASE IN ITALY: PRELIMINARY RESULTS OF A PROSPECTIVE STUDY C.PIATTELLINI1, V.MUNARETTO2, G.REGGIANI2, I.BAIDO2, E.MARAN2, A.BIFFI2, F.BENINI1, A.ZANIN1 AND R.COLOMBATTI2
Authors: C.P. Piattellini, A.Z. Zanin, R.C. Colombatti
Journal: HemaSphere (2023)

Context: Children with sickle cell disease (SCD) suffer from episodes of intense pain such as vaso-occlusive crisis (VOC) that can increase in severity throughout life, leading to a poor quality of life. In addition to epVOCs, patients with SCD can also report recurrent VOCs even on daily basis and chronic pain which can be defined as pain that presents on most days for over over six months. Chronic pain in SCD has profound consequences for the patient: it is often associated with depression and anxiety and can lead to challenges with sleep, and chronic fatigue that impair quality of life. In spite of its burden, chronic pain is difficult to diagnose early for several reasons. In Italy, there is no current data on the prevalence and characteristics of chronic pain in the pediatric population with SCD. Objectives: 1) To evaluate the prevalence and characteristics of chronic pain in a large pediatric, adolescent and young adult SCD cohort (6-24 years) using the American Pain Society Pain Taxonomy (AAPT) Diagnostic Criteria for Chronic Pain in SCD to standardize each patient’s clinical information and using also the PROMIS and PedsQL domains for investigating pain and its effects on health related quality of life (HRQOL); 2)To compare the PROMIS and PedsqL questionnaires to investigate how chronic pain affects quality of life (QoL).A secondary outcome will be to assess if these tools can help to identify potential patients with chronic pain. Methods: The process included multiphase qualitative methodology between the Pain Team and the sCD Team of the AOP. A literature review on SCD was conducted to generate domains of interest for the individual interviews. PROMIS questionnaire investigates Global Health of the patient(7 items),Pain Sensory (8 items),Affective (8 items), Behavior (8 items), Mobility upper extremity (8 items), Strength (4 items), Physical Activity (4 items),Physical stress (4 items), Psychological stress (4 items),Sleep Disturbance (4 items)Sleep Related Impairment (4 items),Anger (5 items) and PedsQL for sickle cell disease. Results: We evaluated 43 patients with SCD ages 6–24 years from May 2021 to September 2022 referred to the Padova SCD reference center.Patients were distributed in 3 classes according to their age (19 (44%) between 6-13 years, 14 (32%) between 13-18 years and 10 (24%) between 18-24 years, 44% were female and only 5 (12%) of them had bone marrow transplants (TMO).Hydroxyurea was treatment regimen of 37 patients, 7 patients among these were on crizanlizumab and 4 subjects in our cohort are on chronic transfusion therapy.According to the American Pain Society Pain Taxonomy (AAPT) criteria for chronic pain in SCD (tab below[1] 16 (37%) patients with presumed chronic pain were identified all between the ages of 13 and 24 years old.Following the scoring of dimensions of Pedsql 12 subjects belong to the category of “excellent quality of life”, 12 to “good quality of life”, 5 to “fair quality of life”, 3to “modest quality of life”, 11 to “poor quality of life. Of the entire patient cohort, 9 fall within the criteria defined by AAPT and thus can be considered to have chronic pain.Further analyses are underway for PROMIS. Conclusions: In our cohort a growing population of SCD adolescents present clinical features of chronic patin These data need to be investigated in further studies thanks a close and muldisciplinary collaboration between Pain services and SCD haematological centers.Reference 1. Dampier C, Palermo TM, Darbari DS, Hassell K, Smith W, Zempsky W. AAPT Diagnostic Criteria for Chronic Sickle Cell Disease Pain. J Pain. 2017 May;18(5):490-498. doi: 10.1016/j.jpain.2016.12.016. Epub 2017 Jan 5. PMID: 28065813.[1]